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| | [[Image:Churg-Strauss syndrome - high mag.jpg|thumb|right|[[Micrograph]] showing a vasculitis. [[H&E stain]].]] |
| This article deals with the '''vasculitides''' (singular ''vasculitis''). Vascular disease that is not vasculitides is covered in the article ''[[vascular disease]]''. | | This article deals with the '''vasculitides''' (singular ''vasculitis''). Vascular disease that is not vasculitides is covered in the article ''[[vascular disease]]''. |
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| *Polyarteritis nodosa (PAN). | | *Polyarteritis nodosa (PAN). |
| *Microscopic polyangiitis. | | *Microscopic polyangiitis. |
| *Wegener's granulomatosis. | | *Granulomatosis with polyangiitis (Wegener's granulomatosis). |
| *Predominantly cutaneous vasculitis. | | *Predominantly cutaneous vasculitis. |
| *Giant cell arteritis (GCA). | | *Giant cell arteritis (GCA). |
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| *Essential cryoglobulinemic vasculitis. | | *Essential cryoglobulinemic vasculitis. |
| *ANCA-associated: | | *ANCA-associated: |
| **[[Wegener's granulomatosis]] (c-ANCA > p-ANCA). | | **[[Granulomatosis with polyangiitis]] (Wegener's granulomatosis) - c-ANCA > p-ANCA. |
| **[[Churg-Strauss syndrome]] (50% ANCA +ve). | | **[[Eosinophilic granulomatosis with polyangiitis]] (Churg-Strauss syndrome) - 50% ANCA +ve. |
| **[[Microscopic polyangiitis]] (usually p-ANCA). | | **[[Microscopic polyangiitis]] - usually p-ANCA. |
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| Notes: | | Notes: |
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| *Giant cell arteritis. | | *Giant cell arteritis. |
| *Takayasu arteritis. | | *Takayasu arteritis. |
| *Churg-Strauss disease. | | *Eosinophilic granulomatosis with polyangiitis (Churg-Strauss disease). |
| *Wegener’s granulomatosis. | | *Granulomatosis with polyangiitis (Wegener’s granulomatosis). |
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| Note: | | Note: |
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| ==Small vessel leukocytoclastic vasculitis== | | ==Small vessel leukocytoclastic vasculitis== |
| *[[AKA]] ''leukocytoclastic vasculitis'', abbreviated ''LCV''. | | *[[AKA]] ''leukocytoclastic vasculitis'', abbreviated ''LCV''. |
| | | {{Main|Small vessel leukocytoclastic vasculitis}} |
| ===General===
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| *Most common cutaneous vasculitis.<ref name=pmid18948765>{{Cite journal | last1 = Brinster | first1 = NK. | title = Dermatopathology for the surgical pathologist: a pattern-based approach to the diagnosis of inflammatory skin disorders (part II). | journal = Adv Anat Pathol | volume = 15 | issue = 6 | pages = 350-69 | month = Nov | year = 2008 | doi = 10.1097/PAP.0b013e31818b1ac6 | PMID = 18948765 }}</ref>
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| Clinical:
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| *Palpable purpura, usu. lower extremity.
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| ===Microscopic===
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| Features:<ref name=pmid18948765/>
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| *Small upper dermis vessels with:
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| *#Neutrophils.
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| *#*Fragmentation of neutrophils (leukocytoclasia).
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| *#Vessel damage: fibrin deposition (bright pink acellular stuff).
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| Has a very broad DDx:<ref name=pmid18948765/>
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| #Infectious:
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| #*Bacterial.
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| #*Viral.
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| #*[[Fungal]].
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| #Vasculitic disorders:
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| #*ANCA mediated vasculitides:
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| #**[[Wegener granulomatosis]].
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| #**[[Churg-Strauss syndrome]].
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| #*[[Henoch–Schönlein purpura]].<ref name=pmid9713395>{{Cite journal | last1 = Kraft | first1 = DM. | last2 = Mckee | first2 = D. | last3 = Scott | first3 = C. | title = Henoch-Schönlein purpura: a review. | journal = Am Fam Physician | volume = 58 | issue = 2 | pages = 405-8, 411 | month = Aug | year = 1998 | doi = | PMID = 9713395 }}</ref>
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| #*Urticarial vasculitis.
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| #Other:
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| #*[[Connective tissue disease]], e.g. [[mixed connective tissue disease]], [[SLE]], [[rheumatoid arthritis]].
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| #*[[Cryoglobulinemia]] - may be due to [[multiple myeloma]], [[hepatitis C]]; have intravascular thrombi.
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| #*Paraneoplastic.
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| #*Drugs.
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| Image:
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| *[http://www.ancavasculitis.com/biopsy.php LCA (ancavasculitis.com)].
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| *[http://path.upmc.edu/cases/case189.html LCA of the GI tract - several images (upmc.edu)].
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| ===Stains===
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| *PAS - look for fungus.
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| ==Microscopic polyangiitis== | | ==Microscopic polyangiitis== |
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| *[http://path.upmc.edu/cases/case260/micro.html Microscopic polyangiitis - several images (upmc.edu)]. | | *[http://path.upmc.edu/cases/case260/micro.html Microscopic polyangiitis - several images (upmc.edu)]. |
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| ==Wegener granulomatosis== | | ==Granulomatosis with polyangiitis== |
| *Abbreviated ''WG''.
| | :Previously known as ''Wegener granulomatosis''. |
| *[[AKA]] ''granulomatosis with polyangiitis''.
| | {{Main|Granulomatosis with polyangiitis}} |
| ===General===
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| *Autoimmune.
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| ====Clinical====
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| *Epistasis.
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| *Renal failure - present as ''nephritic syndrome''.
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| **Renal biopsy: crescentic glomerulonephritis ([[AKA]] [[rapidly progressive glomerulonephritis]]).
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| *[[Pulmonary hemorrhage]].
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| Serology:
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| *c-ANCA +ve.<ref name=Ref_TN2005_RH6>{{Ref TN2005|RH6}}</ref>
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| Notes:
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| *Pulmonary hemorrhage syndromes:<ref name=Ref_PBoD745>{{Ref PBoD|745}}</ref>
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| **[[Goodpasture syndrome]].
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| **Idiopathic pulmonary hemosiderosis.
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| **Vasculitis-assoc. hemorrhage (hypersensitivity angiitis, Wegener granulomatosis).
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| **[[Systemic lupus erythematosus]].
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| ===Microscopic===
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| Features:
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| *Small vessel vasculitis:
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| *#Inflammatory cells within the vessel wall.
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| *#*[[Granuloma]]s - typically poorly formed.<ref name=Ref_PBoD747>{{Ref PBoD|747}}</ref>
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| *#**Multinucleated giant cells - common. (???)
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| *#Vessel wall injury.
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| *#*[[Fibrinoid necrosis]].
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| Notes:
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| *In the lung, the [[granuloma]]s tend to be centrilobular, as the artery travels with the airway and is centrilobular.
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| **It may difficult to find small blood vessels in affected portions of lung.
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| Images:
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| *www:
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| **[http://www.flickr.com/photos/pulmonary_pathology/3734403695/ Wegener's granulomatosis (flickr.com)].
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| **[http://path.upmc.edu/cases/case269.html Wegener's granulomatosis - several crappy images (upmc.edu)].
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| **[http://path.upmc.edu/cases/case660.html Wegener's granulomatosis - more crappy images (upmc.edu)].
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| **[http://path.upmc.edu/cases/case675.html WG - more images (upmc.edu)].
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| *[[WC]]:
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| **[http://commons.wikimedia.org/wiki/File:Wegener%27s_granulomatosis_-b-_intermed_mag.jpg WG - intermed. mag. (WC)].
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| **[http://commons.wikimedia.org/wiki/File:Wegener%27s_granulomatosis_-b-_very_high_mag.jpg WG - fibrinoid necrosis - very high mag. (WC)].
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| ==Churg-Strauss syndrome==
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| *Abbreviated ''CSS''.
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| *AKA ''allergic granulomatous angiitis''.<ref name=emedicine333492>[http://emedicine.medscape.com/article/333492-overview http://emedicine.medscape.com/article/333492-overview]</ref>
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| *[[AKA]] ''Churg-Strauss disease''.
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| ===General===
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| Defining features - memory device ''GAFE'':
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| *'''G'''ranulomata.
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| *'''A'''sthma.
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| *'''F'''ever.
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| *'''E'''osinophilia.
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| Notes:
| | ==Eosinophilic granulomatosis with polyangiitis== |
| *Similar to ''[[Wegener's granulomatosis]]'' (classically c-ANCA +ve) and ''microscopic polyangiitis''.<ref>URL: [http://emedicine.medscape.com/article/334024-overview http://emedicine.medscape.com/article/334024-overview]. Accessed on: 22 January 2011.</ref>
| | :Previously known as ''Churg-Strauss syndrome'' and ''Churg-Strauss disease''. |
| | | {{Main|Eosinophilic granulomatosis with polyangiitis}} |
| ===Microscopic===
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| Features:
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| *Small vessel [[vasculitis]].
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| *Abundant eosinophils.
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| *[[Granuloma]]s.
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| Images:
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| *[http://commons.wikimedia.org/wiki/File:Churg-Strauss_syndrome_-_high_mag.jpg CSS - high mag. (WC)].
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| *[http://commons.wikimedia.org/wiki/File:Churg-Strauss_syndrome_-_very_high_mag.jpg CSS - very high mag. (WC)].
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| DDx:
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| *Eosinophilic vasculitis associated with a [[connective tissue disease]].<ref name=pmid8708015>{{Cite journal | last1 = Chen | first1 = KR. | last2 = Su | first2 = WP. | last3 = Pittelkow | first3 = MR. | last4 = Conn | first4 = DL. | last5 = George | first5 = T. | last6 = Leiferman | first6 = KM. | title = Eosinophilic vasculitis in connective tissue disease. | journal = J Am Acad Dermatol | volume = 35 | issue = 2 Pt 1 | pages = 173-82 | month = Aug | year = 1996 | doi = | PMID = 8708015 }}</ref>
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| =Medium vessel vasculitides= | | =Medium vessel vasculitides= |
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| *Abbreviated ''GCA''. | | *Abbreviated ''GCA''. |
| *[[AKA]] ''temporal arteritis''. | | *[[AKA]] ''temporal arteritis''. |
| ===General===
| | {{Main|Giant cell arteritis}} |
| *Classically afflicts the ''temporal artery''.
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| Clinical features:
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| *Classic finding: jaw claudication, in a patient older than 50 years.
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| *Other findings: headache, vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.
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| Work-up:
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| *CRP, ESR, temporal artery biopsy.
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| **ESR normal (>50 years old): <20 mm/hr males, <30 mm/hr females.<ref>URL: [http://www.nlm.nih.gov/medlineplus/ency/article/003638.htm http://www.nlm.nih.gov/medlineplus/ency/article/003638.htm]. Accessed on: 17 August 2012.</ref>
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| Treatment:
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| *Treat right away with high dose steroids.
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| **Biopsy is confirmatory.
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| ===Microscopic===
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| Features:
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| *Artery with intramural inflammatory cells.
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| **Classically [[granuloma|granulomatous inflammation]].
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| ***Granulomas not required for the diagnosis!
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| *Destruction of arterial wall, e.g. fibrinoid necrosis (pink anucleate arterial wall).
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| Image(s):
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| *[http://www.djo.harvard.edu/files/5077_728.jpg GCA (harvard.edu)].
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| *[http://path.upmc.edu/cases/case646.html GCA - several images (upmc.edu)].
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| ===Sign out===
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| ====Negative====
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| <pre>
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| TEMPORAL ARTERY, LEFT, BIOPSY:
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| - MEDIUM SIZE ARTERY WITHOUT PATHOLOGIC DIAGNOSIS, SEE COMMENT.
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| COMMENT:
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| A negative biopsy does not rule out the possibility of giant cell (temporal)
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| arteritis, as this may be a focal disorder. The clinical management is | |
| dependent upon the clinical impression.
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| </pre>
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| ==Takayasu arteritis== | | ==Takayasu arteritis== |
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| [[Category:Cardiovascular pathology]] | | [[Category:Cardiovascular pathology]] |
| | [[Category:Vasculitides]] |